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进行性外层视网膜坏死综合征

The progressive outer retinal necrosis syndrome.

作者信息

Holland G N

机构信息

UCLA School of Medicine, Jules Stein Eye Institute 90024-7003.

出版信息

Int Ophthalmol. 1994;18(3):163-5. doi: 10.1007/BF00915966.

Abstract

The progressive outer retinal necrosis (PORN) syndrome is a recently described clinical variant of necrotizing herpetic retinopathy in patients with the acquired immunodeficiency syndrome (AIDS). It is caused by varicellazoster virus infection of the retina. Its course and clinical features distinguish it from the acute retinal necrosis syndrome and CMV retinopathy. Early disease is characterized by multifocal deep retinal opacification. Lesions rapidly coalesce and progress to total retinal necrosis over a short period of time. Despite aggressive therapy with intravenous antivirial drugs, prognosis is poor; disease progression and/or recurrence is common, and the majority of patients develop no light perception vision. Total retinal detachments are common. Prophylaxis against retinal detachment using laser retinopexy has not been useful in most cases. PORN syndrome is an uncommon, but devastating complication of AIDS.

摘要

进行性外层视网膜坏死(PORN)综合征是一种最近才被描述的坏死性疱疹性视网膜病变的临床变体,见于获得性免疫缺陷综合征(AIDS)患者。它由水痘-带状疱疹病毒感染视网膜引起。其病程和临床特征使其有别于急性视网膜坏死综合征和巨细胞病毒性视网膜病变。疾病早期的特征是多灶性深层视网膜混浊。病变迅速融合,并在短时间内进展为全视网膜坏死。尽管采用静脉注射抗病毒药物进行积极治疗,预后仍很差;疾病进展和/或复发很常见,大多数患者会发展为无光感视力。视网膜全脱离很常见。在大多数情况下,使用激光视网膜光凝预防视网膜脱离并无效果。PORN综合征是AIDS一种罕见但具有毁灭性的并发症。

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